Progressive multifocal leukoencephalopathy associated with a lymphoproliferative disorder treated with pembrolizumab
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CASE REPORT
Progressive multifocal leukoencephalopathy associated with a lymphoproliferative disorder treated with pembrolizumab Alex Holmes 1,2
&
Tom Wellings 2,3 & Oliver Walsh 4 & Philip Rowlings 1,2
Received: 24 July 2020 / Revised: 14 August 2020 / Accepted: 21 August 2020 # Journal of NeuroVirology, Inc. 2020
Abstract Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease affecting the central nervous system as a result of reactivation of the John Cunningham (JC) polyomavirus and occurs almost exclusively in immunosuppressed individuals. The disease course of PML is variable but usually progressive and often fatal. Treatment is predominantly focused on immune restoration, although this is difficult to do outside of human immunodeficiency virus–associated PML. A recent case series demonstrated a potential role for programmed cell death protein 1 (PD-1) inhibitors, such as pembrolizumab, to contain and/or clear JC virus. Herein, we discuss the first reported Australian case of a 61-year-old female with PML secondary to chemoimmunotherapy demonstrating complete clearance of JC virus as well as clinical and radiological stabilisation following pembrolizumab treatment. Keywords Progressive multifocal leukoencephalopathy . Pembrolizumab . JC virus . Haematologic malignancies
Case presentation A 61-year-old female presented with a 3-week history of progressive homonymous hemianopia and deteriorating visual acuity. History was significant for treatment with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (RCHOP) 14 years previously for diffuse large B cell lymphoma; rituximab monotherapy 8 years previously for low-grade follicular lymphoma; rituximab and bendamustine 3 years previously for relapsed symptomatic low-grade follicular lymphoma; and current treatment with maintenance rituximab and intravenous immunoglobulin for hypogammaglobulinaemia. She underwent magnetic resonance imaging (MRI) of the brain, which demonstrated bilateral occipital asymmetric expansile white matter enhancement consistent with a leukoencephalopathy. Due to concerns for posterior reversible encephalopathy syndrome (PRES)
* Alex Holmes [email protected] 1
Calvary Mater Newcastle, Waratah, NSW, Australia
2
University of Newcastle, Callaghan, Australia
3
John Hunter Hospital, Lambton Heights, NSW, Australia
4
The Canberra Hospital, Garran, ACT, Australia
secondary to hypertension, antihypertensive therapy was commenced. Rituximab and immunoglobulin treatment was ceased. Total T cell count was 640/μL, with CD4 count of 304/μL and CD8 count of 340/uL. CD20 positive B cells were near absent. Lumbar puncture was performed, with cerebrospinal fluid demonstrating lymphocytosis (mixture of T cells with preserved CD4:CD8 ratio) and positive qualitative JC virus nucleic acid by real-time polymerase chain reaction (performed on JCV ELITe MGB assay, ELITechGroup). Plasma JC virus nucleic acid was also detected on the same assay. Visual acuity continued to deteriorate, with gross h
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